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Thymic dysplasia

MedGen UID:
231314
•Concept ID:
C1331541
•
Disease or Syndrome
Synonyms: Thymic Dysplasia; thymic dysplasia
 
Monarch Initiative: MONDO:0004195

Definition

The developmental arrest and architectural distortion of the thymus that results in immunodeficiency. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVThymic dysplasia

Recent clinical studies

Etiology

Retrospective Analysis of 61 Cases of Children Died of Viral Pneumonia.
Chen XB, Du SH, Lu JC, Tan XH, Li DR, Yue X, Wang Q, Wang HJ, Qiao DF
Fa Yi Xue Za Zhi 2020 Apr;36(2):164-168. doi: 10.12116/j.issn.1004-5619.2020.02.003. PMID: 32530160
Helios expression in T-regulatory cells in patients with di George Syndrome.
Klocperk A, Grecová J, Šišmová K, Kayserová J, Froňková E, Šedivá A
J Clin Immunol 2014 Oct;34(7):864-70. Epub 2014 Jul 10 doi: 10.1007/s10875-014-0071-y. PMID: 25008482
Umbilical cord blood transplantation in severe T-cell immunodeficiency disorders: two-year experience.
Knutsen AP, Wall DA
J Clin Immunol 2000 Nov;20(6):466-76. doi: 10.1023/a:1026463900925. PMID: 11202237
Kinetics of T-cell development of umbilical cord blood transplantation in severe T-cell immunodeficiency disorders.
Knutsen AP, Wall DA
J Allergy Clin Immunol 1999 May;103(5 Pt 1):823-32. doi: 10.1016/s0091-6749(99)70426-4. PMID: 10329816
Electrophoresis of lymphoid cells. A study of Bruton type of agammaglobulinaemia, thymic dysplasia, chronic lymphatic leukaemia and of normal human thymocytes.
Wiig JN
Clin Exp Immunol 1975 Jan;19(1):159-65. PMID: 1081923Free PMC Article

Diagnosis

Death from human cytomegalovirus infection in a girl with congenital thymic dysplasia.
Liu Y, Zhu Y, Liu W, Wan C, Guo Q
Virol J 2022 Nov 8;19(1):179. doi: 10.1186/s12985-022-01915-x. PMID: 36348432Free PMC Article
Disseminated Talaromyces marneffei Infection in a Non-HIV Infant With a Homozygous Private Variant of RELB.
Ding X, Huang H, Zhong L, Chen M, Peng F, Zhang B, Cui X, Yang XA
Front Cell Infect Microbiol 2021;11:605589. Epub 2021 Mar 15 doi: 10.3389/fcimb.2021.605589. PMID: 33791233Free PMC Article
Retrospective Analysis of 61 Cases of Children Died of Viral Pneumonia.
Chen XB, Du SH, Lu JC, Tan XH, Li DR, Yue X, Wang Q, Wang HJ, Qiao DF
Fa Yi Xue Za Zhi 2020 Apr;36(2):164-168. doi: 10.12116/j.issn.1004-5619.2020.02.003. PMID: 32530160
A clinical and molecular analysis of a patient with Emanuel syndrome.
Luo JW, Yang H, Tan ZP, Tu M, Luo H, Yang YF, Xie L
Mol Med Rep 2017 Mar;15(3):1348-1352. Epub 2017 Jan 5 doi: 10.3892/mmr.2017.6107. PMID: 28075445
Thymic involution mimicking thymic dysplasia: a consequence of transfusion-induced graft versus host disease in a premature infant.
Seemayer TA, Bolande RP
Arch Pathol Lab Med 1980 Mar;104(3):141-4. PMID: 6892596

Therapy

Death from human cytomegalovirus infection in a girl with congenital thymic dysplasia.
Liu Y, Zhu Y, Liu W, Wan C, Guo Q
Virol J 2022 Nov 8;19(1):179. doi: 10.1186/s12985-022-01915-x. PMID: 36348432Free PMC Article
Umbilical cord blood transplantation in severe T-cell immunodeficiency disorders: two-year experience.
Knutsen AP, Wall DA
J Clin Immunol 2000 Nov;20(6):466-76. doi: 10.1023/a:1026463900925. PMID: 11202237
Usefulness of monoclonal antibodies in the diagnosis and monitoring of patients with primary immunodeficiencies: combined experience in three clinical immunology centers.
Aiuti F, Quinti I, Seminara R, Sirianni MC, Vierucci A, Abo T, Cooper MD
Diagn Immunol 1983;1(3):188-94. PMID: 6388970
Probable graft-vs-graft reaction in an infant after exchange transfusion and marrow transplantation.
Lauer BA, Githens JH, Hayward AR, Conrad PD, Yanagihara RT, Tubergen DG
Pediatrics 1982 Jul;70(1):43-7. PMID: 6806770
Adjuvant chemotherapy of human colorectal adenocarcinoma after growth on mice with congenital thymic dysplasia ("nude"). 2. Tumor remission following treatment with 5-FU and VCR of xenotransplanted primary human colorectal adenocarcinomas.
Schmitz R, Schmitz CA, Hüper J, Richlmayr R
J Cancer Res Clin Oncol 1980;97(1):15-21. doi: 10.1007/BF00411274. PMID: 7400203

Prognosis

Death from human cytomegalovirus infection in a girl with congenital thymic dysplasia.
Liu Y, Zhu Y, Liu W, Wan C, Guo Q
Virol J 2022 Nov 8;19(1):179. doi: 10.1186/s12985-022-01915-x. PMID: 36348432Free PMC Article
Treatment of congenital immune deficiencies with a thymic hormone--thymic humoral factor.
Handzel ZT, Elitsur Y, Algrimawi S, Moses S, Keynan A, Amitai I, Pecht M, Burstein Y, Buchner V, Trainin N
Isr J Med Sci 1986 May;22(5):387-92. PMID: 3528049

Clinical prediction guides

Thymic pathology in primary and secondary immunodeficiencies.
Nezelof C
Histopathology 1992 Dec;21(6):499-511. doi: 10.1111/j.1365-2559.1992.tb00437.x. PMID: 1468748
Thymic involution with loss of Hassall's corpuscles mimicking thymic dysplasia in a child with transfusion-associated graft-versus-host disease.
Gartner JG
Pediatr Pathol 1991 May-Jun;11(3):449-56. doi: 10.3109/15513819109064780. PMID: 1866363
The functional and histological basis for graft-versus-host-induced immunosuppression.
Lapp WS, Ghayur T, Mendes M, Seddik M, Seemayer TA
Immunol Rev 1985 Dec;88:107-33. doi: 10.1111/j.1600-065x.1985.tb01155.x. PMID: 2935484
Thymic involution mimicking thymic dysplasia: a consequence of transfusion-induced graft versus host disease in a premature infant.
Seemayer TA, Bolande RP
Arch Pathol Lab Med 1980 Mar;104(3):141-4. PMID: 6892596
Thymic involution in murine graft-versus-host reaction. Epithelial injury mimicking human thymic dysplasia.
Seemayer TA, Lapp WS, Bolande RP
Am J Pathol 1977 Jul;88(1):119-34. PMID: 18011Free PMC Article

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